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[Protein C deficiency with recurrent infarct of the small intestine]
1Chirurgische Klinik, Universitätsklinikum Heidelberg.
Insights
Protein C deficiency can lead to severe venous thromboses, including mesenteric vein thrombosis. Long-term anticoagulation with dicumarol is an effective treatment for patients with recurrent abdominal issues.
Area of Science:
- Hematology
- Vascular Medicine
Background:
- Protein C and antithrombin III are key inhibitors of the plasmatic coagulation system.
- Clinical significance of Protein C deficiency is increasingly recognized due to advancements in diagnostic assays.
- Familial Protein C deficiency is strongly associated with recurrent venous thrombotic events, often presenting before age 30.
Observation:
- Mesenteric vein thrombosis is a rare but serious manifestation of Protein C deficiency.
- The case highlights a patient with a 10-year history of portal and mesenteric vein thrombosis and recurrent abdominal discomfort.
- Intestinal ischemia necessitated two segmental resections, underscoring the severity of the condition.
Findings:
- Protein C deficiency was diagnosed 10 years after the initial thrombotic event.
- Secondary causes of Protein C deficiency were excluded, confirming a primary genetic defect.
- Long-term anticoagulation with dicumarol was initiated as the primary therapeutic strategy.
Implications:
- This case emphasizes the importance of considering Protein C deficiency in patients with unexplained or recurrent venous thromboses, particularly mesenteric vein thrombosis.
- Early diagnosis and management of Protein C deficiency are crucial to prevent severe complications like intestinal ischemia.
- Long-term anticoagulation strategies, such as dicumarol therapy, can be effective in managing Protein C deficiency-related thrombotic events.
Abstract:
Protein C and antithrombin III represent main inhibitors of the plasmatic coagulation system. Due to the lack of practicable assays the clinical importance of protein C was only established during the last six years. In familial protein C deficiency 77% of patients present with recurrent venous thromboses, half of them below the age of 30. In addition to recurrent superficial thrombophlebitis more serious manifestations like deep vein thrombosis and pulmonary embolism have been described. Mesenteric vein thrombosis has been reported in only 5 cases all of which could be controlled by conservative treatment. In our patient protein C deficiency was discovered 10 years after the angiographic diagnosis of portal and mesenteric vein thrombosis. Thereafter, the patient complained of recurrent abdominal discomfort. Intestinal ischaemia due to mesenteric vein thrombosis required segmental resection twice. Postoperatively the patient was heparinized. After excluding a secondary protein C deficiency due to a lack in vitamin K, hepatic disease, or disseminated intravascular coagulation, long-term anticoagulation by dicumarol was implemented as therapy of first choice.